Bottom Line:
To avoid respiratory complications, the patient was put on a respirator in the ICU for several days after both surgeries.No postoperative complications occurred after both surgeries.We succeeded in treating the patient without complications.

Objective: The authors present the case of a 14-year-old boy with Rubinstein-Taybi syndrome (RSTS) presenting scoliosis.

Summary of background data: There have been no reports on surgery for RSTS presenting scoliosis.

Methods: The patient was referred to our hospital for evaluation of a progressive spinal curvature. A standing anteroposterior spine radiograph at presentation to our hospital revealed an 84-degree right thoracic curve from T6 to T12, along with a 63-degree left lumbar compensatory curve from T12 to L4. We planned a two-staged surgery and decided to fuse from T4 to L4. The first operation was front-back surgery because of the rigidity of the right thoracic curve. The second operation of lumbar anterior discectomy and fusion was arranged 9 months after the first surgery to prevent the crankshaft phenomenon due to his natural course of adolescent growth. To avoid respiratory complications, the patient was put on a respirator in the ICU for several days after both surgeries.

Results: Full-length spine radiographs after the first surgery revealed no instrumentation failure and showed that the right thoracic curve was corrected to 31 degrees and the left lumbar curve was corrected to 34 degrees. No postoperative complications occurred after both surgeries.

Conclusions: We succeeded in treating the patient without complications. Full-length spine standing radiographs at one year after the second operation demonstrated a stable bony arthrodesis with no loss of initial correction.

Mentions:
A 14-year-old boy was referred to our hospital for evaluation of a progressive spinal curvature. He had already been diagnosed with RSTS and noted to have spinal deformity at age one in a regional hospital. Since then he had undergone brace treatment, which failed to halt progression of the scoliosis. There was no mention of either RSTS or scoliosis in his family history. He manifested a peculiar facial appearance, broad thumbs, broad halluces, short stature, and mental retardation (Figure 1 L and R). He was unable to walk without aid, but did not manifest any neurological symptoms such as hyperreflexia or abnormal abdominal reflexes. His general condition was thoroughly examined for any other anomalies before surgery, and we found no airway problems or congenital heart disease. Physical examination revealed a marked right thoracic rib prominence, right shoulder elevation, asymmetric scapulae, and pelvic obliquity. A standing anteroposterior spine radiograph at presentation to our hospital revealed an 84-degree right thoracic curve from T6 to T12, along with a 63-degree left lumbar compensatory curve from T12 to L4. Additional full-length spinal side bending and traction radiographs were obtained to evaluate curve flexibility. On the traction film, the right thoracic curve and the left lumbar curve were corrected to 50 and 42 degrees, respectively (Figure 2 L and R). At the time of surgery, he was 135 centimeters tall and weighed 32 kilograms, and his secondary sex characteristic had not emerged yet. The preoperative films revealed that the triradiate cartilage was open and the Risser grade was 4.

Mentions:
A 14-year-old boy was referred to our hospital for evaluation of a progressive spinal curvature. He had already been diagnosed with RSTS and noted to have spinal deformity at age one in a regional hospital. Since then he had undergone brace treatment, which failed to halt progression of the scoliosis. There was no mention of either RSTS or scoliosis in his family history. He manifested a peculiar facial appearance, broad thumbs, broad halluces, short stature, and mental retardation (Figure 1 L and R). He was unable to walk without aid, but did not manifest any neurological symptoms such as hyperreflexia or abnormal abdominal reflexes. His general condition was thoroughly examined for any other anomalies before surgery, and we found no airway problems or congenital heart disease. Physical examination revealed a marked right thoracic rib prominence, right shoulder elevation, asymmetric scapulae, and pelvic obliquity. A standing anteroposterior spine radiograph at presentation to our hospital revealed an 84-degree right thoracic curve from T6 to T12, along with a 63-degree left lumbar compensatory curve from T12 to L4. Additional full-length spinal side bending and traction radiographs were obtained to evaluate curve flexibility. On the traction film, the right thoracic curve and the left lumbar curve were corrected to 50 and 42 degrees, respectively (Figure 2 L and R). At the time of surgery, he was 135 centimeters tall and weighed 32 kilograms, and his secondary sex characteristic had not emerged yet. The preoperative films revealed that the triradiate cartilage was open and the Risser grade was 4.

Bottom Line:
To avoid respiratory complications, the patient was put on a respirator in the ICU for several days after both surgeries.No postoperative complications occurred after both surgeries.We succeeded in treating the patient without complications.

Objective: The authors present the case of a 14-year-old boy with Rubinstein-Taybi syndrome (RSTS) presenting scoliosis.

Summary of background data: There have been no reports on surgery for RSTS presenting scoliosis.

Methods: The patient was referred to our hospital for evaluation of a progressive spinal curvature. A standing anteroposterior spine radiograph at presentation to our hospital revealed an 84-degree right thoracic curve from T6 to T12, along with a 63-degree left lumbar compensatory curve from T12 to L4. We planned a two-staged surgery and decided to fuse from T4 to L4. The first operation was front-back surgery because of the rigidity of the right thoracic curve. The second operation of lumbar anterior discectomy and fusion was arranged 9 months after the first surgery to prevent the crankshaft phenomenon due to his natural course of adolescent growth. To avoid respiratory complications, the patient was put on a respirator in the ICU for several days after both surgeries.

Results: Full-length spine radiographs after the first surgery revealed no instrumentation failure and showed that the right thoracic curve was corrected to 31 degrees and the left lumbar curve was corrected to 34 degrees. No postoperative complications occurred after both surgeries.

Conclusions: We succeeded in treating the patient without complications. Full-length spine standing radiographs at one year after the second operation demonstrated a stable bony arthrodesis with no loss of initial correction.